This application brings into focus the parallel but separated efforts of many investigators concerned with the pulmonary problems of cystic fibrosis. While maintaining a primary emphasis on the clinical pulmonary problems the SCOR is organized to give maximum interaction and collaboration with basic research studies on pathologic mechanisms of lung injury and the basic metabolic defect. The specific aims of the project are: To use the CLINFO Data Management and Analysis system to coordinate patient care and clinical and basic research studies; to incorporate an on-line library of all the CF literature as a tool to improve clinical care and clinical and basic research; to demonstrate the feasbility and medical effectiveness of home measurements and computer monitoring in clinical care and research; to demonstrate by a model program of newborn screening the incidence of CF, the value and cost of screening and data for accepting or rejecting newborn screening for CF; to determine the interaction between state of nutrition and progression of lung disease in CF subjects; to demonstrate the utility of new pulmonary function tests for preschool CF children and their application for clinical care and clinical research; to elucidate regional changes in lung function and the relationship of these abnormalities to clinical disease and clinical research; to determine the basic disturbance in rheology of mucus and the relationship of clinical ad therapeutic factors to the pulmonary complication of CF; to evaluate all the cellular defense mechanisms of the lung through two related projects, one on the alveolary macrophage and the other on neutrophils and monocytes; and to extend knowledge of the basic metabolic defect in CF.